If not urticaria , what else? - PowerPoint PPT Presentation

By. If not urticaria , what else?. Lamia Elgarhy Assistant Lecturer. Diseases that can mimic Urticaria. Introduction. Urticaria is a common disorder with a complex and not well understood physiopathology.

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If not urticaria, what else?

Urticaria is a common disorder with a complex and not well understood physiopathology.

The main effector cell is the cutaneous mast cell, which can degranulate in response to many different causes (eg, drugs, chemical compounds, autoantibodies, complement factors, or proteases and can release histamine and other mediators that are eventually responsible for wheal formation.

The evaluation of plasma levels of some inflammatory markers (C-reactive protein and interleukin-6) can be useful in identifying patients who are likely to be resistant to antihistamine therapy alone and therefore need systemic steroids to achieve disease control.

Arthropod bite reactions usually appear as fixed pruritic papules, more often but not exclusively on exposed areas.

Papularurticariaappears as fixed, multiple, small erythematous wheals that evolve in pruritic brownish papules, sometimes capped by a vesicle, which eventually become excoriated. (Hypersensitivity reaction)

Lesions more often appear during summer, and are persistent for days or months.

Immediate type localized insect bite reactions are typically urticarial. Lesions are commonly low in number and the patient is aware of the sting. In some instances, serious allergic systemic reactions occur.

A recent study established that the presence of c-kit mutation in all hematopoietic lineages and increased serum beta 2-microglobulin are the most powerful independent markers for predicting the transformation of indolent mastocytosis into a more aggressive form.

Wells syndrome appears clinically as localized or diffuse pruriticerythematous and edematous lesions, which evolve into plaques within a few days and resolve completely, leaving some hyperpigmentation. In some cases, bullous lesions are present.

NEH is a very rare inflammatory dermatosis that presents as solitary or grouped fixed erythematous edematous papules and plaques. The disease is self-limiting, disappearing within days or weeks without scarring

In the vast majority of cases, NEH affects patients with hematologic malignancies—in particular, acute myelogenousleukemiaafterreceiving chemotherapy. In other cases, associated with Behcet’s, or infectious diseases.

The presence of systemic symptoms in particular, fever, asthenia, and arthralgia, and neurologic, respiratory, or cardiovascular signs should alert physicians about the possibility that an urticarial rash is not ordinary urticaria but rather a systemic syndrome with urticaria-like skin lesions.

UV is a small-vessel vasculitiswith predominant skin involvement that represents the main differential diagnosis with chronic urticaria.

It manifests with urticarial skin lesions persisting for more than 24 hours, burning rather than itching, and resolving with hyperpigmentation or bruising. Other skin lesions (purpura or necrosis)may be associated.

Gleich syndrome is also characterized by recurrent episodes of angioedema and urticaria, eosinophilia, elevated serum IgM, fever, and increased body weight; however, it is not associated with internal organ damage.

Autoinflammatory syndromes are a group of heterogeneous monogenic diseases that are characterized by recurrent episodes of multisystemic and seemingly unprovoked inflammation, most commonly caused by an excessive activation of the IL-1b pathway.

Skin lesions, fever, and arthralgia/arthritis are the most common symptoms. Cutaneous lesions frequently have an urticarial appearance, especially in the cryopyrinopathies and in familial Mediterranean fever, whereas erythematousmacules and papules predominate in the other diseases of this group. Skin lesions are temporally associated with relevant systemic symptoms.